IDH3G - isocitrate dehydrogenase (NAD(+)) 3 non-catalytic subunit gamma Gene

中文名称：异柠檬酸脱氢酶 (NAD (+) ) 3 非催化亚基 γ

种属: Homo sapiens

同用名: H-IDHG

关于 IDH3G

功能概要

异柠檬酸脱氢酶催化异柠檬酸氧化脱羧为 2-酮戊二酸。这些酶属于两个不同的亚类，其中一个使用 NAD (+) 作为电子受体，另一个使用 NADP (+) 。已报道了五种异柠檬酸脱氢酶：三种 NAD (+) 依赖性异柠檬酸脱氢酶，定位于线粒体基质，以及两种 NADP (+) 依赖性异柠檬酸脱氢酶，其中一种是线粒体，另一种主要是细胞溶质。 NAD (+) 依赖性异柠檬酸脱氢酶催化三羧酸循环的变构调节限速步骤。每一种同功酶都是由两个α亚基、一个β亚基和一个γ亚基组成的异四聚体。该基因编码的蛋白质是 NAD (+) 依赖性异柠檬酸脱氢酶的一种同工酶的 γ 亚基。该基因是脑室周围异位的候选基因。已经描述了该基因的几种可变剪接的转录本变体，但只确定了它们的一些全长性质。[RefSeq 提供，2008 年 7 月]

Isocitrate dehydrogenases catalyze the oxidative decarboxylation of isocitrate to 2-oxoglutarate. These Enzymes belong to two distinct subclasses, one of which utilizes NAD(+) as the electron acceptor and the Other NADP(+). Five isocitrate dehydrogenases have been reported: three NAD(+)-dependent isocitrate dehydrogenases, which localize to the mitochondrial matrix, and two NADP(+)-dependent isocitrate dehydrogenases, one of which is mitochondrial and the Other predominantly cytosolic. NAD(+)-dependent isocitrate dehydrogenases catalyze the allosterically regulated rate-limiting step of the tricarboxylic acid cycle. Each isozyme is a heterotetramer that is composed of two alpha subunits, one beta subunit, and one gamma subunit. The protein encoded by this gene is the gamma subunit of one isozyme of NAD(+)-dependent isocitrate dehydrogenase. This gene is a candidate gene for periventricular heterotopia. Several alternatively spliced transcript variants of this gene have been described, but only some of their full length natures have been determined. [provided by RefSeq, Jul 2008]